What is ALS?

Amyotrophic lateral sclerosis (ALS), often referred to as “Lou Gehrig’s Disease,” is a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord. Motor neurons reach from the brain to the spinal cord and from the spinal cord to the muscles throughout the body. The progressive degeneration of the motor neurons in ALS eventually leads to their death. When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost. With voluntary muscle action progressively affected, patients in the later stages of the disease may become totally paralyzed.

A-myo-trophic comes from the Greek language. “A” means no or negative. “Myo” refers to muscle, and “Trophic” means nourishment–”No muscle nourishment.” When a muscle has no nourishment, it “atrophies” or wastes away. “Lateral” identifies the areas in a person’s spinal cord where portions of the nerve cells that signal and control the muscles are located. As this area degenerates it leads to scarring or hardening (“sclerosis”) in the region.

As motor neurons degenerate, they can no longer send impulses to the muscle fibers that normally result in muscle movement. Early symptoms of ALS often include increasing muscle weakness, especially involving the arms and legs, speech, swallowing or breathing. When muscles no longer receive the messages from the motor neurons that they require to function, the muscles begin to atrophy (become smaller). Limbs begin to look “thinner” as muscle tissue atrophies.

THE CAUSE OF ALS DISCOVERED – REPORTED AUGUST 21, 2011

SUNDAY, Aug. 21 (HealthDay News) — The apparent discovery of a common cause of all forms of amyotrophic lateral sclerosis (ALS) could give a boost to efforts to find a treatment for the fatal neurodegenerative disease, a new study contends.

Scientists have long struggled to identify the underlying disease process of ALS (also known as Lou Gehrig’s disease) and weren’t even sure that a common disease process was associated with all forms of ALS.

In this new study, Northwestern University researchers said they found that the basis of ALS is a malfunctioning protein recycling system in the neurons of the brain and spinal cord. Efficient recycling of the protein building blocks in the neurons are critical for optimal functioning of the neurons. They become severely damaged when they can’t repair or maintain themselves.

This problem occurs in all three types of ALS: hereditary, sporadic and ALS that targets the brain, the researchers said.

The discovery, published Aug. 21 in the journal Nature, shows that all forms of ALS share an underlying cause and offers a common target for drug therapy, according to the researchers.

“This opens up a whole new field for finding an effective treatment for ALS,” study senior author Dr. Teepu Siddique, of the Davee Department of Neurology and Clinical Neurosciences at Northwestern’s Feinberg School of Medicine, said in a university news release. “We can now test for drugs that would regulate this protein pathway or optimize it, so it functions as it should in a normal state.”

This finding about the breakdown of protein recycling in ALS may also prove useful in the study of other neurodegenerative diseases, specifically Alzheimer’s and other dementias, the Northwestern researchers said.

ALS afflicts an estimated 350,000 people around the world. About 50 percent of patients die within three years of the first symptoms. They progressively lose muscle strength until they’re paralyzed and can’t move, speak, swallow and breathe, the researchers said.